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Phenotypic clusters and survival analyses in interstitial pneumonia with myositis-specific autoantibodies
Yihua Lia1, Yali Fana1, Yuanying Wanga1
1Clinical Center for Interstitial Lung Diseases, Department of Occupational Medicine and Toxicology, Beijing Institute of Respiratory Medicine, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
Background:
Idiopathic inflammatory myopathy (IIM) is highly combined with interstitial pneumonia (IP), often as the initial or solo presentation with positive myositis-specific autoantibodies (MSAs) but does not fulfill the diagnostic criteria.
Objectives:
We aimed to explore the phenotypic clusters and prognosis of the patients with IP and positive MSA, which is called MSA-IP in the present study.
Methods:
A total of 178 patients with MSA-IP were prospectively enrolled for analysis. Serum MSAs were detected using Western blotting. Radiological patterns of IP were determined according to the classification of idiopathic IPs. Clusters of patients with MSA-IP were identified using cluster analysis. Predictors for acute/subacute onset, therapeutic response, IP progression and survival were also analyzed.
Results:
Patients with MSA-IP were classified into four distinct clusters. Cluster 1 were the elderly with chronic onset, nearly normal oxygenation and good survival. Cluster 2 had dyspnea on exertion and nonspecific IP pattern, with moderate survival. Patients in cluster 3 had chronic onset and were prone to IP progression (OR 2.885). Cluster 4 had multi-systemic involvements, positive anti-melanoma differentiation associated gene 5 antibody, and were prone to acute/subacute onset (OR 3.538) and IP progression (OR 5.472), with poor survival. Corticosteroids combined immunosuppressants showed therapeutic response in MSA-IP (OR 4.303) and had a protective effect on IP progression (OR 0.136).
Conclusions:
Four clusters of the patients with MSA-IP suggested the distinct clinical, radiological and prognostic features.
